International Journal of Pediatric Otorhinolaryngology 77 (2013) 1919–1921 Contents lists available at ScienceDirect International Journal of Pediatric Otorhinolaryngology journa l homepage: www.e lsev ier .com/ locate / i jpor l Review Article Intracranial infection caused by spreading through the fossa naviclaris magna – A case report and review of the literature Nili Segal a,*, Ehab Atamne b, Ilan Shelef c, Shorer Zamir d, Daniel Landau d aOtolaryngology Department, Soroka Medical Center, Ben Gurion University, Beer Sheva, Israel b Pediatric Department, Soroka Medical Center, Israel cRadiology Department, Soroka Medical Center, Israel d Pediatric Neurology Unit, Soroka Medical Center, Israel A R T I C L E I N F O Article history: Received 1 July 2013 Accepted 14 September 2013 Available online 23 September 2013 Keywords: Fossa navicularis Clivus Child Intracranial infection A B S T R A C T Fossa navicularis magna is a congenital anomaly representing persistent dehiscence of the basiocciput. This tract from the nasopharynx to the brain is common and carries a potential risk for infection. Nevertheless reports on such dissemination are very rare. We present a child with brain infection via a fossa navicularis magna, the first description to our knowledge, and review the literature on this anomaly. � 2013 Elsevier Ireland Ltd. All rights reserved. Contents 1. Case report . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1919 2. Discussion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1919 3. Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1920 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1921 1. Case report A 12-year-old girl was referred to our emergency room due to fever and headache lasting for oneweek. Her primary care physician treated her with an oral amoxicillin for non-bilious vomiting. On the day of admission, her parents noted a change in her consciousness and worsening headache. Her past medical history reveals asymp- tomatic ventricular septal defect and celiac disease. Physical examination of the girl revealed normal vital signs, left strabismus with positive meningeal signs: neck stiffness, Kernig’s and Brud- zinski’s signs. In addition, herGlasgowcoma scaledecreased from13 on admission to 11 within hours and she developed opistotonus. Empirical intravenous antibiotic treatment was given for suspected bacterial meningitis. A computerized-tomography (CT) scan of the * Corresponding author at: POB 151, Soroka University Medical Center, Beer Sheva, Israel. Tel.: +972 8 6400635/54 4268202. E-mail address:
[email protected] (N. Segal). 0165-5876/$ – see front matter � 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijporl.2013.09.013 head revealed small fluid collection in the nasopharynx just in front of the clivus, air bubbles in clivus and a bony defect in that area (Figs. 1 and 2). A head and neckMRI scan demonstrated a soft tissue swelling in the nasopharyngeal and suboccipital areas, thrombus in the left internal jugular vein with involvement of the sigmoid sinus (Fig. 3). The sphenoid sinus was not opacified. Furthermore, theMRI showed a connection between the upper nasopharynx and the skull base–apatent fossa navicularis. Thegirlwas treatedwithantibiotics as well as anti coagulants and she improved dramatically with almost complete resolution of her strabismus and partial resolution of the venous and sinus thrombosis. 2. Discussion The basiocciput is composed of the sphenoid bone and the occipital bone that fuses between the ages of 13–18 years. The fossa navicularis is a bony dehiscence, which may occur at that region. Several synonyms have been given to that defect in the http://crossmark.crossref.org/dialog/?doi=10.1016/j.ijporl.2013.09.013&domain=pdf http://crossmark.crossref.org/dialog/?doi=10.1016/j.ijporl.2013.09.013&domain=pdf http://dx.doi.org/10.1016/j.ijporl.2013.09.013 mailto:
[email protected] http://www.sciencedirect.com/science/journal/01655876 http://dx.doi.org/10.1016/j.ijporl.2013.09.013 [(Fig._1)TD$FIG] Fig. 1. An axial CT demonstrating air in the clivus bone. [(Fig._2)TD$FIG] Fig. 2. Sagittal CT showing bony dehiscence at the anterior face of the clivus bone – a fossa naviculris. [(Fig._3)TD$FIG] Fig. 3. An axial contrast enhanced T1 MRI with gadolinium demonstrating a filling defect at the right internal jugular vein. N. Segal et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 1919–19211920 literature like fossa pharyngea [1], canalis basilaris medianus [2], fossa navicularis magna and large pharyngeal fossa [3]. Cankal et al. performed an anatomical study and examined 492 dry skulls and reviewed 525 normal CT scans [4]. They found that fossa navicularis exist in 5.3% of the skulls and in 3% of the CT scans. In their study, the patients that had fossa navicularis on CT underwent also MRI scan – no soft tissue was found in the bony defect. They therefore concluded that fossa navicularis has no association with other embryological remnants in this area like Thornwaldt’s cyst. We found only one case report, very similar to ours, describing clivus osteomyelitis in a 5 year old girl probably caused by a spread through a fossa navicularis [5]. The presenting symptoms in that Table 1 Clinical data of patients with infection through a fossa navicularis magna. Demographics Symptoms Signs CT scan Prabhu et al. 5 year old girl Intermittent fever, decreased oral intake, neck pain and neck stiffness Inability to extend the neck fully, bilateral cervical lymphadenopathy Large retropha abscess, cortic destruction of fossa navicula Segal et al. 12 year old girl Fever, headache, neck stiffness Positive meningeal signs, opistotonus, left abducens nerve palsy, anisocoria Mild retropha swelling and small flui air bubbles in aerated sphen fossa navicula child were fever and neck stiffness. Imaging demonstrated retropharyngeal abscess and an osteolytic process involving the clivuswith cortical destruction. In contrast to our patient therewas no evidence of intracranial extension. Table 1 summarizes the two cases. By comparing the only two case reports described in the literature it seems that the common presenting symptoms were fever, neck pains and neck stiffness. Computerized tomography of the previously reported patient showed signs of clivus osteomye- litis and in our patient air within the clivus. Involvement of the bony clivus is uncommon in upper respiratory tract infections or even in retropharyngeal severe infections. We think that clivus involvement should alerts physicians to the rare possibility of fossa navicularis as a route for infection. Our case also demonstrates the possibility of intracranial infection through the defect, which is described for the first time. 3. Conclusion Fossa navicularis is an embryological bony defect in the clivus. Although not uncommon in human skulls it may rarely cause skull MRI Treatment Follow up ryngeal al the clivus, ris Abnormal enhancement of the the clivus and collections within the longus coli muscles, no evidence of intracranial extensions Surgical drainage of the retropharyngeal abscess and antibiotic treatment Asymptomatic within 4 weeks ryngeal d collection, clivus, oid sinus, ris Abnormal enhancement around the clivus (dura and prevertebral space) sinus thrombosis Antibiotic and anticoagulant treatment Very mild abducens paresis, partial resolution of thrombus N. Segal et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 1919–1921 1921 base and even intracranial infections. Fever, neck pains and stiffness with clivus bony involvement on imaging should suggest the diagnosis. References [1] A. Beltramello, G. Puppini, G. El-Dalati, M. Girelli, R. Cerini, A. Sbarbati, et al., Fossa navicularis magna, Am. J. Neuroradiol. 19 (9) (1998) 1796–1798, October. [2] G. Currarino, Canalis basilaris medianus and related defects of the basiocciput, Am. J. Neuroradiol. 9 (1) (1988) 208–211. [3] C. Jacquemin, T.M. Bosley, M. al Saleh, P.Mullaney, Canalis basilarismedianus:MRI, Neuroradiology 42 (2) (2000) 121–123, February. [4] F. Cankal, H.C. Ugur, I. Tekdemir, A. Elhan, T. Karahan, A. Sevim, Fossa navicularis: anatomic variation at the skull base, Clin. Anat. 17 (2) (2004) 118–122, March. [5] S.P. Prabhu, T. Zinkus, A.G. Cheng, R. Rahbar, Clival osteomyelitis resulting from spread of infection through the fossa navicularis magna in a child, Pediatr. Radiol. 39 (9) (2009) 995–998, September. http://refhub.elsevier.com/S0165-5876(13)00460-6/sbref0005 http://refhub.elsevier.com/S0165-5876(13)00460-6/sbref0005 http://refhub.elsevier.com/S0165-5876(13)00460-6/sbref0010 http://refhub.elsevier.com/S0165-5876(13)00460-6/sbref0010 http://refhub.elsevier.com/S0165-5876(13)00460-6/sbref0015 http://refhub.elsevier.com/S0165-5876(13)00460-6/sbref0015 http://refhub.elsevier.com/S0165-5876(13)00460-6/sbref0020 http://refhub.elsevier.com/S0165-5876(13)00460-6/sbref0020 http://refhub.elsevier.com/S0165-5876(13)00460-6/sbref0025 http://refhub.elsevier.com/S0165-5876(13)00460-6/sbref0025 http://refhub.elsevier.com/S0165-5876(13)00460-6/sbref0025 Intracranial infection caused by spreading through the fossa naviclaris magna - A case report and review of the literature Case report Discussion Conclusion References